RESUMO
Kasabach-Merritt phenomenon (KMP) is a rare condition that is associated with two rare vascular tumours: Kapossiform haemangioendothelioma (KHE) and tufted angioma (TA). A 2-year-old girl presented to our emergency room with a haemangioma and respiratory distress. The patient had a violaceous, palpable mass in the right upper chest since she was 5 months old. Severe anaemia, thrombocytopenia, coagulopathy, and hypofibrinogenemia were found. Chest x-ray revealed massive pleural effusion in the right hemithorax. Chest computed tomography (CT) scanning revealed right pleural effusion, multiple destructions of bilateral ribs and multiple osteopenia of thoracic vertebrae. Chest CT angiography revealed a vascular mass in the sternum region. Based on clinical, laboratory and imaging findings, the diagnosis of KMP was established. Clinical, consumptive coagulopathy and thrombocytopenia were resolved by prednisone (3 mg/kg/day) and vincristine (1 mg/body surface area in m2/week) as an adjunct. Unfortunately, she had spontaneous rebleeding and died before the biopsy was done.
